Osteosarcoma, osteogenic sarcoma: symptoms, treatment

Osteosarcoma is considered one of the most frequent varieties of connective tissue tumors. It originates from bone is based on immature cells – osteoblasts that actively divide, forming a tumor of bone tissue.

Among patients with osteogenic sarcoma is dominated by young people, although the tumor may appear in persons of advanced age. The peak incidence occurs in the period between 10-20 years, in boys somewhat later than girls. Up to two times more often the disease is diagnosed in males, typically, by the end of puberty.

Osteosarcoma refers to a very aggressive and rapidly growing tumors that previously actively begins to spread through the blood vessels, giving rise to metastases in the lungs, brain, kidneys and other organs. In connection with this prediction until recently was very poor – only every tenth patient had a chance to survive. In addition, radical surgery was very traumatic, that did not add optimism to people diagnosed with osteosarcoma.

Today, thanks to the efforts of scientists developed as effective methods of treatment of disease, the survival rate increased to 80-90%, but to keep the limb fails the absolute majority of patients.

Causes and characteristics of growth of osteosarcoma

The causes of the disease, as such, remain a mystery. However, this applies to many malignant neoplasms. Long-term observations and the analysis of clinical data allowed, however, to highlight the risk factors that increase the likelihood of developing the disease. These include:

Radiation or chemotherapy in the past in connection with other tumor;

Chronic osteomyelitis;

Paget’s disease accompanied by pathological fractures.

The role of the trauma for a long time debated, as it has been observed that some time after severe damage, fracture, injury was diagnosed and osteosarcoma. However, it has been proved that the injury itself does not lead to the appearance of tumors, but promotes earlier access to a doctor about the pain, and then it turns out that the reason for everything – it is the tumor.

Osteosarcoma occurs more frequently in individuals with benign bone tumors and some tumors with a hereditary character (retinoblastoma). Its occurrence in adolescents and young individuals associated with the intensive growth of the body, the bones are lengthened due to active cell proliferation.

Osteosarcoma most commonly affects the long bones (thigh, shoulder), and flat and short are involved in about 20% of cases. The lower limbs become the target of neoplasia in several times more often than the top, the area of the knee becomes a source of osteosarcoma in 80% of patients. Bone sarcoma of the ribs, scapula, skull – among the rarest and most characteristic sites of the disease, and the edges can become a place of growth, metastatic osteosarcomas.

osteosarcoma of the femur

Osteosarcoma of the femur, without exaggeration, the most common type of the disease. The tumor grows closer to the knee, in the region of the metaphysis that can spread longitudinally, the elements of the articular cavity, can provoke pathological fracture, invasive in the soft tissues and vessels.

Somewhat less often diagnosed with osteogenic sarcoma of tibia, fibula, humerus, the bony framework of the pelvis. Extremely rare localization consider the radial bone, and the defeat of the patella is almost casuistry.

The bones of the skull affects quite rarely, usually in patients – children, and osteosarcoma of the jaws, facial skeleton, in contrast, are more typical for elderly people with prior disfiguring osteodystrophy.

Stages of osteosarcoma

For risk assessment and prognosis of the tumor is important to establish the stage of disease. From the point of view of clinico-anatomical features of osteosarcoma can be localized and metastatic. In the first case it grows in one place in the second – detected metastases. Such a division is somewhat arbitrary, because micrometastases can not simply be diagnosed.

Analysis of the histological type, growth characteristics and the presence of metastasis enables one to identify stages of osteosarcoma:

IA – the tumor is a high degree of differentiation, does not enter blood vessels, has not metastasized.

IIA – the tumor is low grade of differentiation, but limited and not metastatic.

IIB – low-differentiated osteosarcoma without metastasis.

III – is characterized by the appearance of metastases without regard to the degree of differentiation of bone sarcoma.

Manifestations of osteosarcoma

where localized osteosarcoma

The main manifestation of tumors of the bone is considered to be pain. In the initial stages the symptoms are few and non-specific, pain weak and impermanent, reminds rheumatism, arthritis, myositis, therefore, to determine when there is a pathology, problematic.

Soreness characteristic near the joints because of osteogenic sarcoma often grows in the metaphyseal sections of bones. At this stage, the patient can contact the doctor with complaints of joint pain, but studies do not show the presence of pathological changes or effusion.

With increasing tumor mass, increases and the onset of symptoms. The pain becomes constant and unbearable, especially troubling at night, increases with movement, but it is not at rest or immobilization by cast, as is the case with fractures. The intake of painkillers does not bring results, as the tumor continues its rapid growth.

To the point of significant pain syndrome and she changed the affected limb: in the area of growth neoplasia appears thickening and deformation, soft tissues are prone to swelling, appear dilated veins, the skin can become more hot, and when probing is determined by a dense fixed node near the bone and deep soft tissue. The patient can not perform active movements in the joints due to contractures, lame. These symptoms appear about three months from the beginning of tumor growth.

Disease progression is accompanied by an ingrowth of osteosarcoma in soft tissues, it enters cells in the medullar canal, the introduction into the blood vessel walls, which inevitably leads to metastasis. Favoured by the spread of tumor cells becomes blood – vessels. Metastases most often affect the lungs, brain, pleura, kidneys.

osteosarcoma of the jaw, sprouting in the bottom row of teeth

Formation of bones of the skull gives not only pain, but also external cosmetic defect in the form of protrusions and deformation even under small mass of the tumor. In osteosarcoma of the maxilla is disturbed the process of chewing, the jaw is reduced, soft tissue swelling, clearly visible venous blood vessels. Because of the proximity of nerve plexus, Innervate the facial muscles and providing sensitivity in osteosarcoma this localization may be paresthesia and paresis of the muscles.

Osteosarcoma of the lower jaw before the upper, gives strong pain. Tissue swollen gums, teeth become loose, the characteristic itching and pain in the oral cavity. Movement of jaw is very painful.

Osteogenic sarcoma considered a tumor very early on and actively metastatic. By the time of diagnosis in every fifth patient has signs of a lung that is visible on the radiograph, and up to 80% of patients are microscopic metastases diagnosed on CT scan. Lymphogenous spread is rarely observed, but if it occurs, the prognosis is significantly worse.

In advanced stages of osteosarcoma, in addition to local, it is bound to be common manifestations of the disease – weight loss, fever due to tumor toxicity, fatigue, loss of appetite.

Video: how does bone sarcoma

How to detect osteosarcoma?

Unfortunately, it is not uncommon for the diagnosis of osteosarcoma is exposed on the late stage and treatment does not have the expected result. This is due to the paucity of symptoms in the early stages, fuzzy pain syndrome, late address to the doctor.

The appearance is unclear, suspicious pain in the bones or joints, which are enhanced and not removed pain, a visit to a specialist is better not to postpone. The first to suspect a tumor, can become a surgeon or a trauma surgeon, who will thoroughly investigate the nature of complaints and the dynamics of symptoms.

It is important to clarify, it is not tolerated if the patient has been exposure in the past, because the tumor can occur decades after radiation. The lack of swelling or varicose veins is not a sign talking about the absence of tumors.

osteosarcoma of the knee joint on x-ray

The probable osteosarcoma not do without additional research. Are required to be:

Radiography of the proposed place of neoplasia;

CT, MRI to clarify the boundaries of the tumor and condition of the surrounding tissues;

Ultrasound of the abdomen, chest x-ray to exclude metastatic growth;

Biopsy tissue tumors under a microscope.

All, without exception, patients undergo a General and biochemical blood tests, urine test. In the early stages, significant changes may not be, but by the time the tumor into the surrounding tissue in the blood test will be increased leukocytes, elevated ESR, in the late stages with a chance of anemia.

Before the operation to verify the state of vessels and the presence of tumor cells is performed angiography. This method of research determines the course and scope of intervention. If osteosarcoma cells already in the blood vessels, organoboranes operation will not be possible.

Features of treatment of bone sarcomas

In the treatment of osteosarcoma must be taken into account localization, the prevalence of the tumor, the presence or absence of metastases, and sensitivity to specific chemotherapy drugs. The main methods, effective with this type of tumors, recognized as operation and chemotherapy.

The scheme of treatment of patients with osteosarcoma involves preoperative chemotherapy, then surgery, and then chemotherapy again. Preoperative treatment intended to reduce the volumes of neoplasia and destruction of small foci of metastasis. In addition, this approach allows the oncologist to adjust the sensitivity to specific drugs that will increase the effectiveness of subsequent chemotherapy after surgical treatment.

In the case of osteosarcoma are assigned to methotrexate at high doses, ifosfamide, doxorubicin, cisplatin, adriablastin. Preoperative chemotherapy is called neoadjuvant. If necessary, in subsequent names and dosages may be changed.

In the postoperative period are assigned up to ten courses of treatment (adjuvant chemotherapy), including those drugs that are marked as the best answer before the surgery. Adjuvant chemotherapy is required for the destruction of microscopic metastases, or treatment of larger lesions, if any has formed.

Surgical treatment – the main stage, the efficiency and timeliness which is determined by the prognosis of the disease. More recently, in osteosarcoma were carried out mostly amputations and assertequal, after which the patient lost part or all of a limb. Such interventions were very traumatic and risky, because you removed a large volume of tissue.

Today, doctors are trying to save the patient the greatest possible activity and functionality by conducting a sparing, organ-saving resection. Of course, in advanced stages, such operations may be not shown due to active tumor growth and metastasis, but with timely diagnosis of the tumor, they are the method of choice.

Organ-saving operations for osteosarcoma include the excision of fragment of bone or joint, after which the defect is replaced by a prosthesis made of artificial materials, your own or cadaver bone. Thus, the limb can be saved, and the patient can lead a normal life. Moreover, even detected metastases may not be a barrier to conserving surgery.

Organ-preserving surgery

It happens that the tumor has gone so far that the surgeon has no choice but to spend an extended radical surgery, indications for which consider a large osteosarcoma, germination of its main vessel and nerves, the vastness of the lesion with necrosis and hemorrhage, the reproduction of microorganisms, pathological fractures.

Considered radical amputation with removal of all limbs and joints assertequal. Advanced include ipodstore-abdominal disarticulation and interscapular-thoracic amputation. They are very traumatic and require a long rehabilitation on the background of postoperative chemotherapy.

Surgical removal may be subject and secondary foci osteosarcoma. Most often they are found in the lungs. If chemotherapy is effective against primary tumors, large metastases can simply resect surgically.

Radiation therapy has no independent value in osteosarcoma as the tumor is insensitive to radiation. However, this method is used in such patients in whom surgery is impossible to produce in connection with the peculiarities of localization of the neoplasm, severe General condition of the organism, concomitant diseases, preventing the intervention.

Experience has shown that organ-sparing surgery in the majority are not inferior in efficacy of the radical, and the addition of chemotherapy before and after the intervention significantly improves prognosis and survival. In addition, of great importance to the quality of life of the patient is the preservation of the limb without compromising the radicalism of the treatment, and that is at least 80% of patients.

After the treatment of osteosarcoma is test examination, including CT or MRI of the affected area, x-ray examination of the lungs, bone scintigraphy, ultrasound of the abdomen to exclude metastasis.

The prognosis for osteosarcoma pretty good, given the aggressiveness of the disease. So, the consistent use of chemotherapy, surgery, and again chemotherapy 5-year survival rate at 70% with a limited form of the disease. If the tumor is sensitive to chemotherapy, this figure reaches 80, and even 90%. Such high rates of survival associated with new treatment approaches and the use of highly effective medicines, but more recently it was the amputation and survived only every tenth patient.

If you were able to completely remove the tumor during surgery, and foci of metastasis decreased under the action of chemotherapeutic agents, the prognosis will be better.

For assessing the prognosis of patients with osteosarcoma is conventionally divided into three groups:

The group with high risk when the volume exceeds 150ml neoplasia and chemotherapy of tumor cells either not die, or more than half of them remain viable.

The low-risk group – when osteosarcoma does not exceed 70 ml in volume, it does not matter its sensitivity to chemotherapy and the survival rate is more than 90%.

Group standard risk are all other patients, the survival rate is about 65-67%.

The intensity and duration of treatment is determined by the risk group, which can be attributed to a specific patient. After going through all the stages of treatment, the patient was excluded from the field of view of oncologists, because the risk of metastasis and recurrence of the disease remains. For monitoring it would need to visit the doctor every three months for the first two years after treatment, the third year, the specialist examines the patient every four months, following two years – once in 6 months and then annually. When nizkobonitetnyh tumors to visit a specialist less often: every six months during the first two years, then annually.